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SS- Heme > Hemolytic Anemias > Flashcards

Hemolytic Anemias Flashcards

1
Q

hemolytic anemia

A

intravascular rupture of RBC or increased uptake by phagocytes

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2
Q

plasma

A

liquid portion of blood

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3
Q

serum

A

liquid left over if you let blood clot

CANNOT asses blood coagulation

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4
Q

hemolytic anemia lab findings

A
  1. free hemoglobin increased
  2. LDH increased
  3. haptoglobin reduced (infection/inflammation can artificially increase)
  4. unconjugated bilirubin
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5
Q

spherocytosis

A

genetic hemolytic anemia
defects membrane anchors, spectrin, or ankyrin
Dx: osmotic fragility test

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6
Q

hemoglobinopathies

A

genetic hemolytic anemia: crystallization of hemoglobin
missense mutation
Hgb S, HgB C, HgB SC, Hgb E
Dx: hemoglobin electrophoresis

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7
Q

pyruvate kinase deficiency

A

genetic hemolytic anemia
decreases in ATP inhibits the Na/K ATPase, sodium leaks into the cell, water follows and cell bursts
polychromes counted as reticulocytes
Dx: enzyme activity test

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8
Q

hereditary spherocytosis

A

genetic hemolytic anemia
defects membrane anchors, spectrin, or ankyrin
Dx: osmotic fragility test

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9
Q

pyropoikilocytosis

A

genetic hemolytic anemia
defects membrane anchors, spectrin, or ankyrin
Dx: osmotic fragility test

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10
Q

hereditary eliptocytosis

A

genetic hemolytic anemia
defects membrane anchors, spectrin, or ankyrin
Dx: osmotic fragility test

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11
Q

G6PD deficiency

A

genetic hemolytic anemia
X-linked
defect in anti-oxidant system: can’t produce NADPH via the pentose phosphate pathway to reduce glutathione needed to reduce peroxide
Heinz bodies, methemoglobinemia
induced by: fava beans, bactrim (sulfamethoxazole), anti-malarials, lots of drugs
Dx: enzyme activity

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12
Q

methemoglobinemia

A

extensive oxidation of heme iron (now Fe3+)

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13
Q

Drugs that should be avoided in G6PD deficiency

A
  1. dapsone
  2. methylthioninium chloride (methylene blue)
  3. nitrofurantoin
  4. phenazopyridine
  5. primaquine
  6. rasburicae
  7. tolonium chloride (toluidine blue)
    * Many used to treat malaria and G6PD is prevalent in same region
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14
Q

paroxysmal nocturnal hemoglobinuria

A

neoplastic/ acquired genetic
unimpaired complement fixation: MAC destroys RBC (no phosphotidylinosital glycan: no DAF)
Dx: flow cytometry
Tx: allogenic bone marrow transplant and ecluzimab

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15
Q

malaria

A

infectious hemolytic anemia
intracellular protozoal parasite
travel to tropical/ subtropical areas: anopheles mosquito
high fever, dark urine, jaundice
thick smear needed if parasite load isn’t high

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16
Q

merozoites

A

early form of plasmodium

17
Q

plasmodium falciparum

A

parasite that causes malaria
most lethal form
Africa

18
Q

plasmodium vivax

A

parasite that causes malaria

Caribbean

19
Q

babesia

A

infectious hemolytic anemia
protozoal intracellulair parasite
ticks
NE U.S.

20
Q

Bartonella baciliformis

A

infectious hemolytic anemia (sever in acute phase)
rash in chronic phase
fever, splenomegaly
sand flies
northern Andes (Peru, Ecuador, Colombia)
other names: Carrions disease, Oroya fever

21
Q

C. perfringens (welchii)

A

infectious hemolytic anemia
normal skin flora
gas gangrene or sever anemia
trauma, septic abortions, rare complication of cholecystitis, or cancer

22
Q

warm autoimmune hemolytic anemia

A

Ab against RBC antigens: cleared by phagocytes
extravascular and intravascular hemolysis
microspherocytes, nucleated red cells, basophilic stippling, polychromasia, hemolysis lab tests
DAT positive at 37 F for complement and Ab
NO blasts or myelocytes
Associated with: lymphoma, malignancies, autoimmune
Tx: steroids, splenectomy
Prognosis: poor

23
Q

extravascular hemolysis

A

macrophages clear RBC marked with C3b

24
Q

intravascular hemolysis

A

MAC complex destroy RBCs

25
Q

direct antiglobulin test (DAT)

A

direct Coombs test

26
Q

Why would you add exogenous RBCs to a DAT?

A

RBC with Ab bound may get lysed/cleared rapidly and undetectable to DAT

27
Q

What is a problem with DAT?

A

nonspecific: up to 8% of hospitalized patients have a positive DAT

28
Q

cold autoimmune hemolytic anemia

A

cold agglutinins with broad thermal amplitude (up to 37 F) or high concentration: IgM Ab
raynaud’s phenomenon, hemolysis by serum tests, RBC agglutination, polychromasia, basophilic stippling, nucleated RBC
Clumping: may get inaccurately low red cell count and hematocrit: have to warm up for accurate count
DAT: + at room temp for IgM, + at 37 F for C3
Ab screen: + at room temp, - at 37 F
NO blasts or myleocytes
associated with: viral syndromes, Mycoplasma
Tx: steroids, splenectomy
Prognosis: chronic, seasonal

29
Q

thrombotic thrombocytopenia purpura (TTP)

A

Ab against adamTS13
thromboses, thrombocytopenia, hemorrhage
microangiopathic hemolytic anemia: scistocytes
fever, renal failure, CNS symptoms in absence of sepsis
Tx: plasmapheresis

30
Q

AdamTS13

A

cleaves vWF to the appropriate size

31
Q

vWF

A

link platelets to damaged endothelium

32
Q

blood transfusion: major antigen mismatch

A

acute hemolysis immediately or within hours

33
Q

blood transfusion: minor antigen mismatch

A

delayed hemolytic reactions (1-7 days)

SS- Heme (13 decks)

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